tag:blogger.com,1999:blog-20742459881801524532018-02-17T01:39:51.561-08:00Say What?leahhttp://www.blogger.com/profile/05004783118268323560noreply@blogger.comBlogger739125tag:blogger.com,1999:blog-2074245988180152453.post-11953829528267700972016-12-15T09:25:00.000-08:002016-12-15T09:25:04.412-08:00One Week with a Cochlear Implant<div style="text-align: center;"><a href="https://3.bp.blogspot.com/-cBhgxixNkg0/WFLN6wezH0I/AAAAAAAAEv4/8ZV2rQDFpGcO5U6pF47R74vwHTogcocOwCLcB/s1600/december14.jpg" imageanchor="1"><img border="0" height="320" src="https://3.bp.blogspot.com/-cBhgxixNkg0/WFLN6wezH0I/AAAAAAAAEv4/8ZV2rQDFpGcO5U6pF47R74vwHTogcocOwCLcB/s320/december14.jpg" width="177" /></a></div><div style="text-align: left;"><br /></div><div style="text-align: left;">The initial phases of having a cochlear implant requires a lot of programming to adjust amount of current going to each electrode. This process is called MAPping, and Nolan will be going back to NYU frequently in December to get new MAPs. We went back this Tuesday-Wednesday to fine tune his hearing.</div><div style="text-align: left;"><br /></div><div style="text-align: left;">He has adjusted very rapidly to the cochlear implant and prefers it to his hearing aid, though he definitely does better in noise when he has two ears to hear (and his hearing aid is required for school to verify he is getting the best signal possible).&nbsp;</div><div style="text-align: left;"><br /></div><div style="text-align: left;">Within 48 hours of switch-on, he was playing the piano. He states the sound is a little bit different, with emphasis on the "little bit." This is spectacular, as we were concerned the piano might sound significantly different to our budding pianist.&nbsp;</div><div style="text-align: left;"><br /></div><div style="text-align: left;"><br /></div><iframe allowfullscreen="" frameborder="0" height="315" src="https://www.youtube.com/embed/1ydyDFpMWak" width="560"></iframe> <br />A week later, he declares the sound as "normal" and happily plays the piano. We are impressed and did not expect the adjustment to happen so quickly.<br /><br />He was also able to hear in the water for the first time. He loves the Neptune for this purpose. The Neptune cannot do all of the things the Naida can (noise programs, etc.) but it is perfect for water use. It does require a slightly stronger MAP than his Naida to get the same sound quality for Nolan.<br /><br /><br /><iframe allowfullscreen="" frameborder="0" height="315" src="https://www.youtube.com/embed/CNi-2GuktO8" width="560"></iframe> <br /><br />We drove back to NYU on Tuesday after working and going to school for half a day. Nolan had a rough start in the morning as he was tired and is not a morning person. MAPping was a little difficult because he would point to a 5 (out of 10) for sound volume - the target is a 6 - and then point to a 4 when his audiologist increased the sound. This is inconsistent so we did some MAPping with live voice and found a good program for him. Talking to him later, he wanted it "louder" so when she would increase the volume, he would point to a 4 to make her increase it even more. We told him that he should be consistent with the number he chooses and not make it relative - hopefully our next MAPping session will be a little easier.<br /><br />His new MAP is fantastic and he was given two different MAPs to use - one at a stronger level than the other. He set himself to the second program by the time we went over for his speech assessment.<br /><br />The AVT was thrilled with his progress and obtaining a cochlear implant was clearly the right decision for Nolan. His speech understanding has soared from 36% best aided to over 90% in just one week. This is not a formal assessment since he has not been tested in the booth yet, but informally he understands most single-syllable words without speech reading using his implant alone. He won't need to be seen by the speech/language department again at NYU until we're at a year post-implant. We will be going to University Hospitals in Cleveland to have an assessment and determine what we should work on.<br /><br />We were going to pick up his new Link hearing aid on Tuesday, but decided to hold off until our next session. Since Nolan still has 2 MAPs loaded and the hearing aid can only hold one program, we will wait until the 27th when Nolan has just one MAP loaded.<br /><br />He has loved his "noise" program, which allows him to hear whoever he is facing and blocks out background noise. We also added an "echo" program for use in the gym, where reverberation is an issue. So he has four programs - MAP 1 (which he is not using as it is too quiet already), MAP 2 (the program he is on most of the time), Program 3 (noise) and Program 4 (gym/reverberation).<br /><br />We are glad we have a little break from traveling back and forth to New York City. We will go back on the 27th and get another MAP and his hearing aid.dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com0tag:blogger.com,1999:blog-2074245988180152453.post-64799539838639468042016-12-11T17:37:00.000-08:002016-12-11T18:09:36.872-08:00Cochlear Implant Activation Day<div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-VfardV1OtZ8/WE3y-7-hZ6I/AAAAAAAAEts/jTHcGUZx5fMOBegoJTuWArHO74aoLdTQgCLcB/s1600/IMG_1926.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-VfardV1OtZ8/WE3y-7-hZ6I/AAAAAAAAEts/jTHcGUZx5fMOBegoJTuWArHO74aoLdTQgCLcB/s320/IMG_1926.JPG" width="213" /></a></div><br />We drove into NYC a day early, because NYU was amazing and managed to squeeze in Nolan's post-op appointment on the Monday before his activation. We stayed in Secaucus this time, because hotel rates in the city at Christmastime are ridiculous. The hotel was great, and we drove to the mall near the Newport Station and took the PATH train in from Jersey City. There was some traffic, but it wasn't horrific and was definitely worth the savings in hotel fees.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-GMnQH98s0Jo/WE30q9uadFI/AAAAAAAAEt4/uuGlS4WDoDQnfdMceFahGWx4-VFynZfdACLcB/s1600/jerseytraffic.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="180" src="https://1.bp.blogspot.com/-GMnQH98s0Jo/WE30q9uadFI/AAAAAAAAEt4/uuGlS4WDoDQnfdMceFahGWx4-VFynZfdACLcB/s320/jerseytraffic.jpg" width="320" /></a></div><br />Nolan's appointment wasn't until 3:00 pm, so we drove to Hoboken to check out the Cake Boss bakery. We were able to meet <a href="http://thelawrencetriplets.blogspot.com/">this really awesome family</a> while we were in town. It was really cool to meet everyone in person, and my boys LOVED Cormac, Colin, and Ciaran.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-wdw1KigGb2w/WE31aL6FVnI/AAAAAAAAEt8/zG6agA-Nm3owBAb9eaSVGDXNkGMSYgeCACLcB/s1600/carlosbakery.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="180" src="https://4.bp.blogspot.com/-wdw1KigGb2w/WE31aL6FVnI/AAAAAAAAEt8/zG6agA-Nm3owBAb9eaSVGDXNkGMSYgeCACLcB/s320/carlosbakery.jpg" width="320" /></a></div><br />We wanted to do something fun that all five boys would enjoy, and the Liberty Science Center was closed on Mondays. We ended up going to a place called HudsonPlay, which has an indoor Ninja Warrior style obstacle course. It was kid heaven.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://3.bp.blogspot.com/-b5xJyIdWVc4/WE32G59JOzI/AAAAAAAAEuA/TeKiYQcP7aAoPLj4itQATzWlSg0uWx3LQCLcB/s1600/IMG_1896.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://3.bp.blogspot.com/-b5xJyIdWVc4/WE32G59JOzI/AAAAAAAAEuA/TeKiYQcP7aAoPLj4itQATzWlSg0uWx3LQCLcB/s320/IMG_1896.JPG" width="320" /></a></div><br />Nolan's post-op was at 3:00 pm and we were on a tight schedule to make it, so we ate at McDonald's and caught the PATH train to 33rd street. We made it into the Otology department <i>just in time</i>. We were taken back and Nolan was seen by the surgeon, who said everything looks as it should. Nolan still can't swim for a little while and we have to use Ciprodex ear drops for another week, but this is all normal for a recovering tympanoplasty. You can't even see the scar behind his ear - the surgical technique is amazing.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-MEau8GIrHc0/WE330T1LsGI/AAAAAAAAEuI/oKTJBi6IwDMMf0B-xjeeNd59MZItFVFCwCLcB/s1600/NYUview.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-MEau8GIrHc0/WE330T1LsGI/AAAAAAAAEuI/oKTJBi6IwDMMf0B-xjeeNd59MZItFVFCwCLcB/s320/NYUview.jpg" width="175" /></a></div><br />The appointment was over quickly, and we left to explore the city. We wanted to see Rockefeller Center decked out for the holidays, so we walked over and enjoyed looking at the lights. The boys enjoyed going into the Lego store and the Nintendo store. We decided to go back to Jersey City for dinner, and ate at Orale - living in Western NY, we are always desperate for great Mexican food.<br /><br />It was nearly 11:00 pm by the time we settled into bed that night. We had walked a lot and everyone was exhausted, so we had no issues with falling asleep. The activity of the day was fantastic to wear everyone out!<br /><br />We were up early on Tuesday, since we needed to drive through traffic and get into the city during rush hour. We didn't have to wait long before we were called back for Nolan's activation. They did an initial check on his implant and electrode #11 wasn't working, but we were told losing one electrode wasn't a huge deal. Still, I was disappointed - I hated losing even one electrode. Fortunately, the issue was caused by an "air bubble" and disappeared when current was run through the array. Big sigh of relief! All 16 electrodes were up and running.<br /><br />Volume levels were tested (we couldn't hear these tests - they were all inside Nolan's head) and once set, the microphone was turned on.<br /><br />He was so nonchalant about it - and it exceeded our wildest expectations. We expected him to hear "whistles and beeps" on the first day, but he was able to understand open-set words within the first two minutes.<br /><br /><div class="separator" style="clear: both; text-align: center;"><iframe allowfullscreen="" frameborder="0" height="315" src="https://www.youtube.com/embed/_MNRcIZzkNQ" width="560"></iframe></div><br />He was given four programs that day - the first three with increasing levels of volume and the fourth one was quieter, in case the volume was too much for him on that first day. Within 30 minutes of leaving the appointment, he was on the highest volume level.<br /><br />We wandered around Union Square and the Christmas market and had lunch at Max Brenner (the chocolate is to die for). Nolan's battery wasn't fully charged from the factory settings and died in about 2 hours. We were all really tired, so we opted to head back to the hotel to re-charge the battery and relax.<br /><br />Nolan has been able to describe the sounds he is hearing. All voices sound much lower-pitched to him, so I sound like a man with a deep voice. Dennis is unintelligible and has a voice too low to register. Crunching leaves sound like high-pitched dinging. Clapping and water running sounds normal. He has an echo of sound on some low-frequency consonants, especially the "D" sound (this should resolve with MAPping). He is also refusing his hearing aid, so we know it isn't from the acoustic hearing in his left ear (that ear is severe so there isn't a lot of sound coming in without a hearing aid).<br /><br /><iframe width="459" height="344" src="https://www.youtube.com/embed/3RrEet8lA3M" frameborder="0" allowFullScreen=""></iframe><br />The next day, we returned to the cochlear implant center and Nolan was given four new programs. The first three were of increasing volume levels again, and the fourth one was a noise program. Nolan can use that program to "tune out" background noise and will only hear the person he is facing. We also picked up Nolan's new Neptune processor, which is totally waterproof and can be used for swimming, snow activities, and in heavy rain.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-DCqJDiH9hPA/WE37bppRCjI/AAAAAAAAEus/f5mleoEgVPsReldT9xyiedSbyuJeSflHwCLcB/s1600/neptune.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-DCqJDiH9hPA/WE37bppRCjI/AAAAAAAAEus/f5mleoEgVPsReldT9xyiedSbyuJeSflHwCLcB/s320/neptune.jpg" width="175" /></a></div><br />Before heading out on the long drive home, we went to the National Museum of Mathematics. They have many hands-on activities and it was a perfect stop for a couple of hours.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-UoE5SqaXSVg/WE38EXozsvI/AAAAAAAAEu0/ZMQsfpSyriMqAOx4NxB_U_vYoAW6jtjJwCLcB/s1600/IMG_1946.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://1.bp.blogspot.com/-UoE5SqaXSVg/WE38EXozsvI/AAAAAAAAEu0/ZMQsfpSyriMqAOx4NxB_U_vYoAW6jtjJwCLcB/s320/IMG_1946.JPG" width="320" /></a></div><br />I couldn't believe how well Nolan was hearing with his implant - just one day after activation. He could hear me on the car ride home without looking at me - despite the road noise.<br /><br /><iframe allowfullscreen="" frameborder="0" height="315" src="https://www.youtube.com/embed/1sMWPs1xCCI" width="560"></iframe> <br /><br />He has been activated for 5 days now and his progress is absolutely astounding - but that is another blog post. We are over the moon with how well he has adjusted to the cochlear implant, though now our battle lies in getting him to continue to wear the hearing aid in his other ear. We insist for school hours, but when he has the option, he removes it and prefers the implant by far.dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com0tag:blogger.com,1999:blog-2074245988180152453.post-52055579289834416192016-11-14T16:02:00.000-08:002016-11-14T16:03:25.324-08:00Surgery DayLiving far from the cochlear implant center means a significant amount of planning is involved for each appointment. For Nolan's surgery, the boys' grandma flew in to take care of Matt while we were in New York City. Surgery was planned for Friday (Veteran's Day), but we needed to leave on Thursday. The boys had a half day of school on Thursday, so the plan was to let Nolan go to school and leave directly once school was dismissed.<br /><br />Unfortunately, that same day, <a href="http://www.post-journal.com/news/page-one/2016/11/city-police-looking-for-person-of-interest-in-south-side-shooting/">a man shot his wife and went on the lam</a> in our local area, causing all of the schools to go on lock-down. The schools are fantastic about keeping our children safe, but this was quite stressful as we worried about where the man could be and wondered how long the lock-down would last. In addition, Dennis is leaving for a business trip in China shortly and was trying to get Visa documentation through as the trip and the Visa deadline are in close proximity. I managed to get the boys and Dennis managed to fax the necessary paperwork through by 3:00 pm, which is when we left. We made it to our hotel in Secaucus, NJ at 10:00 pm - which was a pretty fast trip! The benefit of leaving later was the total lack of traffic late at night.<br /><br />Nolan's check-in time was 8:00 am for a 10:00 surgery, and we knew we needed to get through the Lincoln Tunnel. We left early and hoped the traffic wouldn't be too horrible - it wasn't and we made it to the hospital early.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-hVthYsLUYo0/WCpI6o0ysSI/AAAAAAAAEsM/1OgGY3zT0dYb0Ydaoj7SCo22GPXkHX17wCLcB/s1600/drivetonyusurgery.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="180" src="https://4.bp.blogspot.com/-hVthYsLUYo0/WCpI6o0ysSI/AAAAAAAAEsM/1OgGY3zT0dYb0Ydaoj7SCo22GPXkHX17wCLcB/s320/drivetonyusurgery.jpg" width="320" /></a></div><div style="text-align: center;">&nbsp;<i>Driving into NYC in the morning</i></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-_XMicImbf9Q/WCpI6nLq5aI/AAAAAAAAEsQ/dWpMliFptXkH1vXth93sIbNbIr8FlBGjgCLcB/s1600/Nolangoingtonyusurgerycar.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://1.bp.blogspot.com/-_XMicImbf9Q/WCpI6nLq5aI/AAAAAAAAEsQ/dWpMliFptXkH1vXth93sIbNbIr8FlBGjgCLcB/s320/Nolangoingtonyusurgerycar.jpg" width="176" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Nolan is not a morning person</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We checked in and they took Nolan back immediately - which was fantastic. It wasn't long before he was weighed, checked, and allowed to watch a little T.V.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-L0d-E5Qv__8/WCpJQHclqoI/AAAAAAAAEsU/mwuIxpTqfOMVvr7pwfAVps55OrG7DO7AQCLcB/s1600/waitingpreop.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://4.bp.blogspot.com/-L0d-E5Qv__8/WCpJQHclqoI/AAAAAAAAEsU/mwuIxpTqfOMVvr7pwfAVps55OrG7DO7AQCLcB/s320/waitingpreop.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Watching college football</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">The anesthesiologist stopped by and we went over his history, and we opted for the pre-surgical Versed to help ease any anxiety prior to surgery. Nolan can sometimes become very anxious prior to surgery due to his past experiences, so the Versed was a good choice. It did help him remain relaxed through the rest of the morning.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">Dr. Roland stopped by to mark the ear undergoing implantation, and Nolan was dressed for surgery and ready to go.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://3.bp.blogspot.com/-4Lj7Ng7cb1g/WCpJoA0VA-I/AAAAAAAAEsY/Ot4GYEYSlAM9tWGkdZSXpNpgseSD9be6QCLcB/s1600/goingonagurney.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://3.bp.blogspot.com/-4Lj7Ng7cb1g/WCpJoA0VA-I/AAAAAAAAEsY/Ot4GYEYSlAM9tWGkdZSXpNpgseSD9be6QCLcB/s320/goingonagurney.jpg" width="176" /></a></div><div style="text-align: center;">&nbsp;<i>Ready to go</i></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://3.bp.blogspot.com/-f3PMNl598XY/WCpJoOhbzGI/AAAAAAAAEsc/M5HsFHpB98MzSDW5AGJqTFUQ8amrswNFwCLcB/s1600/rightearmarked.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://3.bp.blogspot.com/-f3PMNl598XY/WCpJoOhbzGI/AAAAAAAAEsc/M5HsFHpB98MzSDW5AGJqTFUQ8amrswNFwCLcB/s320/rightearmarked.jpg" width="176" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Marking the ear</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">I was allowed to go back with him and he was soon asleep. Dennis and I went down to the lobby (Child Life is fantastic at NYU and escorted us down to the lobby to show us where the cafe was located). We ate quickly and then went back to the waiting room, where we were updated frequently as to Nolan's status. The surgery took about an hour and a half, and Dr. Roland came out to tell us the tympanoplasty was done and the cochlear implant was in place and working perfectly. Everything went as smoothly as it could have gone.&nbsp;</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">It took a little while to see our guy, as the recovery room was a little overcrowded that Friday. They moved him to recovery and we finally saw him at around noon. He was given a little extra fentanyl as he was in some pain at that point of the day.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://3.bp.blogspot.com/-udVVDp8u7iE/WCpKc6mxOGI/AAAAAAAAEsg/smFWNwjm7AMaeORIqm3VrdnOglo-GuPNgCLcB/s1600/justoutofsurgery.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="180" src="https://3.bp.blogspot.com/-udVVDp8u7iE/WCpKc6mxOGI/AAAAAAAAEsg/smFWNwjm7AMaeORIqm3VrdnOglo-GuPNgCLcB/s320/justoutofsurgery.jpg" width="320" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Sweet buddy</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">For other parents who are going through the same thing, the first couple of hours in recovery were pretty rough. Nolan would wake up whimpering and was in considerable pain. He received a little more fentanyl and then returned to sleep. We had to do this twice, and it was hard to see him struggling those first couple of hours. We are very grateful to the nurses, who took fantastic care of him and also recommended the fentanyl, as when he finally woke up from the final dose, he was MUCH better.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">By 3:00 pm (just four hours post-surgery), he was awake, talking, hungry, and thirsty. The turn-around was dramatic and he was doing amazingly well! He drank a good deal of ice water. He requested a popsicle. Unfortunately, there were no popsicles to be found. He did get a peanut butter and jelly sandwich, but continued to ask for a popsicle. Child Life stopped by to check on him (did I mention how wonderful they are?) and immediately went on a popsicle-hunting mission. A few minutes later, he was all set.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://3.bp.blogspot.com/-vX1LOHVdrdg/WCpLbK6aP2I/AAAAAAAAEss/i9k6Hg4u10EPhulXpPr6ZLHDZ5MmuQ4eACEw/s1600/popsicle.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="180" src="https://3.bp.blogspot.com/-vX1LOHVdrdg/WCpLbK6aP2I/AAAAAAAAEss/i9k6Hg4u10EPhulXpPr6ZLHDZ5MmuQ4eACEw/s320/popsicle.jpg" width="320" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Popsicles make everything better</i></div><div class="separator" style="clear: both; text-align: center;"></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">Child Life also gave him this adorable teddy bear - so cute!</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-85-p7TWkScs/WCpLkxxX2GI/AAAAAAAAEsw/59A0jjQxPwAe9sWrNGlrzkoqsYRgIhV3ACLcB/s1600/stuffedbear.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://4.bp.blogspot.com/-85-p7TWkScs/WCpLkxxX2GI/AAAAAAAAEsw/59A0jjQxPwAe9sWrNGlrzkoqsYRgIhV3ACLcB/s320/stuffedbear.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>A friendly bear is always good</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">Nolan was doing well and was released at 4:00 pm. Right into rush hour traffic in Manhattan. Oh, and the new security measures around Trump Tower. And also? Trump protesters. A perfect storm of awfulness for driving in a city that is known for its "normal" congestion.&nbsp;</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-KHXbKjGQLAQ/WCpL36BuX4I/AAAAAAAAEs0/T-LB75-sXZUIwpxziPRCDxei33bzFZOHQCLcB/s1600/leavingmanhattan.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-KHXbKjGQLAQ/WCpL36BuX4I/AAAAAAAAEs0/T-LB75-sXZUIwpxziPRCDxei33bzFZOHQCLcB/s320/leavingmanhattan.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Manhattan traffic chaos</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We needed to get to the Lincoln Tunnel. We wanted to get OFF THE ISLAND. So many streets were blocked and we could not get down many of the streets that would lead to the Lincoln Tunnel. In 1.5 hours, we had gone three miles.. in a circle. It was a nightmare! Nolan was a real trooper and was entertained by his Kindle in the backseat. He was really hungry, but we were stuck in traffic and there was nothing we could do.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We <i>finally</i>&nbsp;managed to make it to the Lincoln Tunnel. I was never so happy to see a tunnel in my life!</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-L_cqX6qrBdY/WCpMbaiunTI/AAAAAAAAEs8/YAJPf9-mlOokVFtAT_KIxsraZCz6JsFNwCLcB/s1600/lincolntunnelseeit.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://1.bp.blogspot.com/-L_cqX6qrBdY/WCpMbaiunTI/AAAAAAAAEs8/YAJPf9-mlOokVFtAT_KIxsraZCz6JsFNwCLcB/s320/lincolntunnelseeit.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>O, Lincoln Tunnel, I adore you</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We made it to our hotel and Nolan was so hungry he was nearly in tears. We walked into the lobby, but it turns out the chef had called in sick that day so there was no food available at the hotel. With the traffic outside, we couldn't get to the restaurants on the other side of the freeway, so we went to the only restaurant within walking distance from the Hilton Garden Inn - a Dominican food place. The food was good, but Nolan was really tired and decided he wasn't very hungry after all.&nbsp;</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-dBY5wSONlAw/WCpM7NVvzoI/AAAAAAAAEtA/xUzilVD6cvQ05c5zlAztHuI84uNPkM1kACLcB/s1600/dominicanfood.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-dBY5wSONlAw/WCpM7NVvzoI/AAAAAAAAEtA/xUzilVD6cvQ05c5zlAztHuI84uNPkM1kACLcB/s320/dominicanfood.jpg" width="176" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Not very hungry after all</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">After dinner, I carried him back to our hotel room and Dennis went to the car to get our supplies. Nolan was really wiped out, but was absolutely delighted to find a cookie bouquet waiting for him in the room <a href="http://thelawrencetriplets.blogspot.com/">from this amazing family.</a>&nbsp;</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-UZQGqSfEkg4/WCpNc4jLosI/AAAAAAAAEtE/QTBC7BZ_zvcQGb0inizPq3LjikJo6KVFgCLcB/s1600/cookies.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-UZQGqSfEkg4/WCpNc4jLosI/AAAAAAAAEtE/QTBC7BZ_zvcQGb0inizPq3LjikJo6KVFgCLcB/s320/cookies.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">After a really long and stressful day, it was such a sweet and lovely thing to find!</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">I managed to get Nolan into his pajamas and he settled in to watch Shrek on the television. He seemed to be OK and fell asleep around 9 pm.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-gHJelbLRbPE/WCpNzjNw1QI/AAAAAAAAEtI/TEWw5-DdrA8rOUpAI3PhMmX9EopYr-HJwCLcB/s1600/inthehotelroom.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://4.bp.blogspot.com/-gHJelbLRbPE/WCpNzjNw1QI/AAAAAAAAEtI/TEWw5-DdrA8rOUpAI3PhMmX9EopYr-HJwCLcB/s320/inthehotelroom.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Resting after a long day</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">He was really unsteady and dizzy that evening, so I was grateful that he settled in and fell asleep. He did wake frequently through the night, but we finally just turned on the TV and allowed him to watch it as needed throughout the night. We don't allow much television at home, so this was a treat for him.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We were able to remove his bandage in the morning. I wasn't sure what to do about this, as we didn't have scissors with us. We tried using toenail clippers, but we were never going to get through it with clippers! We found scissors at the front desk and managed to snip it off.&nbsp;</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-1OVFL0lOcU8/WCpOYc1XQfI/AAAAAAAAEtQ/xPjBfXJcglAFJECF6mt5h2rJT7HYReglwCLcB/s1600/leavingforhome.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-1OVFL0lOcU8/WCpOYc1XQfI/AAAAAAAAEtQ/xPjBfXJcglAFJECF6mt5h2rJT7HYReglwCLcB/s320/leavingforhome.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Ready to go home.</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We were very grateful to head home. We stopped for breakfast somewhere in the Poconos and for lunch in Corning, NY. We made it home by 3:00 pm.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">Nolan has been doing really well, though he is sore and still a little dizzy. Every day seems to be a little better. He is doing really well this evening and is a lot more talkative and energetic, so I think he will probably be close to "normal" tomorrow.&nbsp;</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">He has been loving the special presents and treats. His aunt and uncle sent him a massive teddy bear, and he is pretty happy about the gift.</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-K3TXZeezgZ8/WCpOzec8HzI/AAAAAAAAEtU/Wbe9L3-7aQoNI2O6EWOTkkVvkuzH70FUQCLcB/s1600/giantbear.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://4.bp.blogspot.com/-K3TXZeezgZ8/WCpOzec8HzI/AAAAAAAAEtU/Wbe9L3-7aQoNI2O6EWOTkkVvkuzH70FUQCLcB/s320/giantbear.jpg" width="175" /></a></div><div class="separator" style="clear: both; text-align: center;"><i>Giant bears are full of awesomeness</i></div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">I am sure he will be back to school soon, though I am worried about roughhousing or Nolan falling, so we may keep him home this week. He has two half-days this week, anyway, so I'd rather he be fully recovered before sending him back to the germ-filled school (strep and everything else is going around at the moment).</div><div class="separator" style="clear: both; text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: left;">We only have three weeks until activation day. I can't believe how quickly time is flying! We are very excited for him to get his new "ear!"</div>dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com1tag:blogger.com,1999:blog-2074245988180152453.post-65912312452087996882016-09-28T18:58:00.000-07:002016-09-28T18:58:20.659-07:00Surgery Date Set<div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-RNp2WLfpr2o/V-xx9wL8UTI/AAAAAAAAEro/Fcj3ZHqsjQcDGfPJh63kTEc428P9ccoDQCLcB/s1600/IMG_0646.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://4.bp.blogspot.com/-RNp2WLfpr2o/V-xx9wL8UTI/AAAAAAAAEro/Fcj3ZHqsjQcDGfPJh63kTEc428P9ccoDQCLcB/s320/IMG_0646.JPG" width="320" /></a></div><div style="text-align: center;"><i>Playing in Brooklyn</i></div><br />We returned to the Big Apple to see the surgeon with regard to Nolan's cochlear implant. The hearing tests already demonstrated he would be a good candidate for the implant, but this appointment was to determine if he was a good candidate medically. The staff at NYU is just amazing - truly, they are world-class. Dr. R. looked at Nolan's MRI and CT scan results from when he was a baby (normal anatomy). His anatomy is good, so there will be no issues with the device placement.<br /><br />The one thing we were worried about is the gaping hole in Nolan's right eardrum. We have been told the hole is about 15% to 20% of his eardrum, and I had <i>no</i>&nbsp;idea what they were going to do about that. Would they need to do a second surgery to seal the eardrum? Would that preclude him from candidacy? I truly had no idea, and was hopeful it wouldn't be that big of a deal.<br /><br />Fortunately, this is NYU and they are amazing, so there will just be <i>one</i>&nbsp;surgery and Nolan will have his tympanoplasty when the cochlear implant is placed. I am extremely grateful this will be a "one and done" surgery. This will also prevent more infections from happening to that ear, as most infections arise <i>because</i>&nbsp;he has a massive hole in his eardrum. We are very careful to keep that ear dry, but it will be a relief not to worry about water in the ear anymore.<br /><br />Surgery is scheduled for November 11, which is fantastic as Nolan will miss less school (Veteran's Day Holiday). He will obviously miss some school the week after surgery, but we are trying to keep the missed days to a minimum.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-ei7iPiE7Ry0/V-xz0toTPPI/AAAAAAAAEr0/wbv1ho3FErY_fy9iNPJDT-CfUgncz3X8QCLcB/s1600/NYUmedcenter.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://1.bp.blogspot.com/-ei7iPiE7Ry0/V-xz0toTPPI/AAAAAAAAEr0/wbv1ho3FErY_fy9iNPJDT-CfUgncz3X8QCLcB/s320/NYUmedcenter.jpg" width="176" /></a></div><div style="text-align: center;"><i>NYU &nbsp;Medical Center</i></div><div style="text-align: center;"><i><br /></i></div>He will be "switched on" December 6 and December 7. He will get his first MAP (program) on December 14. &nbsp;Hearing with a cochlear implant is a process, and the programming has to be adjusted frequently in the initial stages. The sound will likely be strange for a while, but with time and therapy his brain will adjust and the sound will normalize.<br /><br />We drove home immediately after the appointment and made it home by 1:00 am. Fortunately we won't return until November 10, so we have some downtime before the crazy travel schedule hits again!<br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com3tag:blogger.com,1999:blog-2074245988180152453.post-24921318736155131192016-09-24T15:48:00.000-07:002016-09-24T15:48:20.602-07:00Goodbye, Hearing - Hello, NYU<div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-p5trxhz1TyQ/V-b4mJaJwSI/AAAAAAAAEqk/m7E4g-koHZgrc0stzWUteHKmIjwOCxGzACLcB/s1600/IMG_0493.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://1.bp.blogspot.com/-p5trxhz1TyQ/V-b4mJaJwSI/AAAAAAAAEqk/m7E4g-koHZgrc0stzWUteHKmIjwOCxGzACLcB/s320/IMG_0493.JPG" width="320" /></a></div><br />Life is insanely busy and I don't often update Nolan's blog - but here is my yearly post (ha). Nolan is now 9 years old. We have had an insanely cool summer, visiting Quebec City, Tadoussac to see beluga whales in the wild, and hiking the via ferrata in Ausable Chasm.<br /><br /><div style="text-align: center;"><a href="http://1.bp.blogspot.com/-E_jrY4tIX10/V-b5RV0fj0I/AAAAAAAAEqs/l4HhJe45OmsJfmxA58qhkbZYDq3f3YzHACK4B/s1600/IMG_9632.JPG" imageanchor="1"><img border="0" height="320" src="https://1.bp.blogspot.com/-E_jrY4tIX10/V-b5RV0fj0I/AAAAAAAAEqs/l4HhJe45OmsJfmxA58qhkbZYDq3f3YzHACK4B/s320/IMG_9632.JPG" width="213" /></a></div><br />Last December, we went to Cleveland Clinic to evaluate Nolan's hearing. At the time, there were some small changes in his left ear (his good ear), but the right ear was largely stable, ranging from 60-90 dB (moderate to severe). His aided thresholds were in the mild-moderate range.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-194mZ4ni1NA/V-b5wNb-sPI/AAAAAAAAEq4/zwHXZ5WeRZIV0PE6bnRvXdkQkVsC7l2MwCLcB/s1600/clevelandclinicDecember2015.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="179" src="https://2.bp.blogspot.com/-194mZ4ni1NA/V-b5wNb-sPI/AAAAAAAAEq4/zwHXZ5WeRZIV0PE6bnRvXdkQkVsC7l2MwCLcB/s320/clevelandclinicDecember2015.jpg" width="320" /></a></div><br />His speech perception using live voice was 80% in the "bad" ear and 100% in the good ear. We knew we would need to upgrade his hearing aids from Bolero Q SP's if his hearing degenerated anymore, but also knew that if his right ear lost more, he might be in the range for a cochlear implant.<br /><br />Over the summer, we were getting more "whats" and non-responses from Nolan. If he was looking at you, he could understand you. If he wasn't looking at you, he sometimes wouldn't detect that you had spoken. The left side still seemed to be getting sound, but the right ear appeared to have great difficulty. I did the Ling sounds for just the right ear, and the only sound he picked up was "ah."<br /><br />I knew this meant he had lost additional hearing, and made the call to NYU's cochlear implant program. I wanted an evaluation to get him into the system just in case the right ear had dropped. If he didn't qualify, that would be fine - but I had suspicions that his right ear was losing residual hearing quickly.<br /><br />They reviewed his audiogram from last December and said he wouldn't be a candidate with that audiogram, but considering his history we should bring him in for an evaluation. We had hotels booked and made the 7 hour drive to New York City (this drive takes longer than 7 hours with traffic, but is still well worth the trip).<br /><br />NYU performed a full cochlear implant evaluation for Nolan. We first met with the LSLS (Listening and Spoken Language Specialist) and the audiologist who would be testing Nolan's hearing. We explained that we knew he was not a likely candidate, but preferred to err on the side of caution since he had been having difficulty lately and is in the fourth grade - we don't want his academics to suffer if the left ear decides to take a sudden dive and we lose his "good" ear.<br /><br />The testing was broken up into three days. Each day, Nolan sat in the booth for 90 minutes and performed various listening tasks. Day 1 was the unaided audiogram. I don't have a copy of the audiogram yet, but my suspicions were confirmed. The left ear is largely where it was at Cleveland Clinic (though his speech reception has declined slightly), but the right ear is now profoundly deaf. His best threshold was at 90 dB in the low frequencies, dropping to over 100 dB in the highs (to no response at 8000 Hz). Nolan's speech reception was 48% in the good ear with speech at 85 dB, and was very poor in the right ear (24% at 95 dB).<br /><br />Suddenly we were no longer "getting into the system," but entering true cochlear implant candidacy evaluations. Cochlear implant candidacy requires a lot more than unaided testing, so we would return the following day for testing in the aided condition.<br /><br />Nolan really wanted to see the Statue of Liberty while we were in NYC, so we left the clinic to explore Manhattan.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-q8D8Q0Tm9fU/V-b9cgmktoI/AAAAAAAAErE/k7tR0B2E95YAME_OGZt0s40Q3LGhoVrUwCLcB/s1600/IMG_0461.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://4.bp.blogspot.com/-q8D8Q0Tm9fU/V-b9cgmktoI/AAAAAAAAErE/k7tR0B2E95YAME_OGZt0s40Q3LGhoVrUwCLcB/s320/IMG_0461.JPG" width="320" /></a></div><div style="text-align: center;">&nbsp;Taking a cruise to see Lady Liberty</div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-cn-HqCmcxXg/V-b9gPnw0iI/AAAAAAAAErM/0qWBmXxWy84xMqn_iuwD9zBKdFY3X1WdwCLcB/s1600/IMG_0505.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://4.bp.blogspot.com/-cn-HqCmcxXg/V-b9gPnw0iI/AAAAAAAAErM/0qWBmXxWy84xMqn_iuwD9zBKdFY3X1WdwCLcB/s320/IMG_0505.JPG" width="320" /></a></div><div style="text-align: center;">At the World Trade Center on September 12&nbsp;</div><div style="text-align: center;"><br /></div><div style="text-align: left;">We returned to the cochlear implant clinic the following day to repeat a lot of the previous testing, this time with Nolan's hearing aids on. The audiologist said words and sentences, but this time the volume was at 60 dB instead of 95 dB, since he had hearing aids on. His left (good) ear was fantastic - he was able to get a score of 84% in that ear. The right ear, unfortunately, only scored 36% for words and 27% for phonemes, even with a hearing aid on. His right ear, even in the perfect silence of the sound booth, just wasn't really hearing clear speech. His candidacy looked like a surer thing, but they wanted to try one more thing first: more &nbsp;powerful hearing aids.</div><div style="text-align: left;"><br /></div><div style="text-align: left;">Nolan has super power aids which are supposed to accommodate a loss of up to 110 dB in the high frequencies, but he was at or beyond that level unaided. We needed to determine if the Naida UP (the most powerful hearing aid on the market) would bring his aided hearing into the desired range.&nbsp;</div><div style="text-align: left;"><br /></div><div style="text-align: left;">We left the clinic to explore more of New York City, including the Natural History Museum.&nbsp;</div><div style="text-align: left;"><br /></div><div class="separator" style="clear: both; text-align: center;"><a href="https://2.bp.blogspot.com/-t_0q-lMPvPg/V-b-2f-hK9I/AAAAAAAAErU/9N2COMCjpPIqRNPjP2GTTq2zn6T008c7gCLcB/s1600/IMG_0545.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://2.bp.blogspot.com/-t_0q-lMPvPg/V-b-2f-hK9I/AAAAAAAAErU/9N2COMCjpPIqRNPjP2GTTq2zn6T008c7gCLcB/s320/IMG_0545.JPG" width="320" /></a></div><div style="text-align: center;">Dinosaurs!</div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://4.bp.blogspot.com/-rXRvNOKOq-E/V-b9dNZGC3I/AAAAAAAAErI/bdlnWPQghUQcNJs7td23aTr1JmoSS0qRwCLcB/s1600/IMG_0529.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="https://4.bp.blogspot.com/-rXRvNOKOq-E/V-b9dNZGC3I/AAAAAAAAErI/bdlnWPQghUQcNJs7td23aTr1JmoSS0qRwCLcB/s320/IMG_0529.JPG" width="320" /></a></div><div style="text-align: center;">Checking out Times Square</div><div style="text-align: left;"><br /></div><div style="text-align: left;">The third day of testing would be the determining factor for whether or not Nolan would qualify for an implant. Nolan had language testing performed (his language skills are insanely high, due to very early intervention and <s> a mother that never shuts up </s>&nbsp; extensive exposure in the home environment.<br /><br />He was brought back into the booth, this time with the Naida UP's. The UP hearing aid brought his left ear into the mild range for hearing levels and he obtained 100% speech recognition with it. We were thrilled! The right ear, however, did not fare as well. He was aided to the mild/moderate range with the Naida UP in the right ear. He did improve his speech understanding with the Naida UP, but was still missing half of the speech signal, at 60 dB in quiet (live voice).<br /><br />This is a tricky situation - as he <i>might</i>&nbsp;do well with the Naida UP hearing aids, but his hearing loss is progressive, the right ear is already profound, and if we continue to wait the left ear will likely drop - leaving him with very poor hearing while trying to navigate the fourth grade. It is also likely the right ear will continue to drop, so we would be spending a significant amount of money (near $8000) for a very brief period of time. There is no telling when the left ear will drop, but it always trails the right ear by a few months to a year. We decided that if he was a candidate, we would proceed with implantation of the right ear. This will give him a "stable" ear (once rehabilitated) in case the left ear decides to take a dive on him in the future.<br /><br />It was decided that Nolan would be a candidate for a cochlear implant in the right ear, and we were able to look at the three major brands who manufacture the device. Each brand is slightly different, but all serve the same function and operate in the same manner. It is a bit like buying a car - all are reliable and will get you to your destination, but the features vary from model to model. We took home a ton of literature from that appointment and returned to our home in Western NY to figure out which brand we want our son to have - for the rest of his life. While we know they all work just fine (cochlear implant centers wouldn't use a brand that didn't work), we need to determine what the best brand would be <i>for Nolan</i>.<br /><br />We are returning to NYU tomorrow to meet with Nolan's surgeon. We are bringing Nolan's CT scan and MRI from when he was a baby so we won't have to repeat those tests. The process is long and involved, and we have to get insurance approval, the surgery scheduled, turn on the device, program the device, and do a lot of auditory-verbal-therapy to help his brain learn to use the signal generated by the implant. While it will take some time for him to adjust to it, his hearing will be better with the cochlear implant than it will be with his hearing aid.&nbsp;</div><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com1tag:blogger.com,1999:blog-2074245988180152453.post-66191699834653481362015-11-03T13:29:00.002-08:002015-11-03T13:31:15.001-08:00It's Been a Long Time<div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-IHf046SGAKg/Vjke3Rn7XLI/AAAAAAAAEpE/y3t2e_I8_CY/s1600/IMG_5058blackandwhite.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://3.bp.blogspot.com/-IHf046SGAKg/Vjke3Rn7XLI/AAAAAAAAEpE/y3t2e_I8_CY/s320/IMG_5058blackandwhite.JPG" width="213" /></a></div><br />I haven't updated Nolan's blog in a long time. Primarily because I now work outside the home (and have little time for writing), and also because things have been going "hunky-dory." There is some exciting news on the home front: Nolan is growing on his own and we will be able to remove his feeding tube later this month. This is a wonderful development and we are absolutely thrilled. He'll never be a giant, but he is growing and otherwise thriving.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-7u0H3En8jeM/VjkgCCeXvqI/AAAAAAAAEpQ/qybEFyWi60g/s1600/IMG_3855.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://2.bp.blogspot.com/-7u0H3En8jeM/VjkgCCeXvqI/AAAAAAAAEpQ/qybEFyWi60g/s320/IMG_3855.JPG" width="320" /></a></div><br />He plays soccer and loves it. He plays the piano and is flying through his lesson books - he has a natural talent and his fingers fly over the keyboard. He does wonderfully in the academic arena and is excelling in his third grade class.<br /><br />His hearing, however, continues to deteriorate. This past spring, we had his PE tubes (grommets) removed and paper patches applied to try to heal his eardrums. Unfortunately, the right ear is stubborn with its infections and he had an infection in the recovery period. The paper patch didn't "take" in that ear.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-H8QxpwJu1Ks/VjkjkVA2S3I/AAAAAAAAEpc/Y-FOmqE1xxA/s1600/myringoplasty.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://1.bp.blogspot.com/-H8QxpwJu1Ks/VjkjkVA2S3I/AAAAAAAAEpc/Y-FOmqE1xxA/s320/myringoplasty.jpg" width="179" /></a></div><br /><br />The ear infections continue in that ear. It is a catch-22: they won't do a tympanoplasty in that ear until it is "dry," but the infections are not likely to stop, particularly since he has such a large hole in that eardrum. The nurse practitioner estimated about 15%-20% of his eardrum is missing. We had a hearing test done in an infection-free time and found the following:<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-aDV6YxbgH7s/VjkkE5gm2ZI/AAAAAAAAEpk/wgUxthPDxfs/s1600/audiogram.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://4.bp.blogspot.com/-aDV6YxbgH7s/VjkkE5gm2ZI/AAAAAAAAEpk/wgUxthPDxfs/s320/audiogram.jpg" width="213" /></a></div><br /><br />The right ear is now in the severe range from 1000 Hz-8000 Hz. He does hit moderate at 500 Hz in that ear, but all other frequencies are in the severe range. The left ear is doing better, at a moderate-moderately severe level across the board. The conductive component is still there. The ENT does not believe it is due to the hole in his eardrum, though we cannot be sure. Our current ENT has decided to ignore the right ear and simply focus on the better ear. His better ear (the left one) is aiding to 25-35 dB (the "As" on the audiogram above). Our current audiology practice does not test the aided ears separately, so the worse ear is not evaluated.<br /><br />Unfortunately, we are seeing some signs of speech "slippage" from Nolan. His high frequency sounds are less clear. Words like "kits" are becoming "kiss." He currently has (yet another) ear infection in the right ear. Buffalo cultures it each time, delaying the treatment time and causing that ear to continue to degenerate.<br /><br />Since the audiology practices locally don't test the aided ears individually, it is hard to tell what, if anything, the right ear is perceiving. When we do the Ling 6 test with the right ear aided (taking out the left hearing aid), he only detects the "AH" sound.<br /><br /><iframe allowfullscreen="" frameborder="0" height="315" src="https://www.youtube.com/embed/uw3OopJQWSQ" width="420"></iframe> <br />I am concerned about the right ear "dropping off the map," so I contacted Cleveland Clinic for a second opinion. The audiology practices in Buffalo are not practicing according to the current best practice guidelines for pediatric audiology, and Nolan is not aided as well as he should be.<br /><br />Cleveland Clinic is amazingly responsive and have a plan in place. We have an appointment for December 21 with audiology and the ENT surgeon there: we will see if there is anything that can be done for the right ear (since there is a perforation in the eardrum) and we will see what can be done with his aided hearing results in the sound booth. Cleveland does real-ear testing (Nolan has never had real-ear testing) and will test each aided ear individually. There are several options available to us, and the ultimate goal is to allow Nolan to hear better. It is possible his hearing aids can be cranked up to allow him to access more sound, or we may need a technology change.<br /><br />In any case, there is our update (hey, it's only been nearly two years)!<br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com1tag:blogger.com,1999:blog-2074245988180152453.post-91248354668049064712014-01-19T13:59:00.000-08:002014-01-19T13:59:08.504-08:00New GlassesThe optometrist's shop must have rushed Nolan's new eyeglasses frames after they ordered the wrong ones. Within 24 hours, we received a call stating they were ready to be picked up!<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-fTWMYaudJWo/UtxKGCW4BCI/AAAAAAAAEZw/HXi53W0qPqw/s1600/003.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://1.bp.blogspot.com/-fTWMYaudJWo/UtxKGCW4BCI/AAAAAAAAEZw/HXi53W0qPqw/s1600/003.JPG" height="213" width="320" /></a></div><br />He is so excited to have his new glasses. He picked out an orange case and was thrilled to wear his new specs to school. These are definitely the right frames, and Nolan is pleased that the arms match his ski helmet (both are green).<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-8543c5R3Lro/UtxKaCLhblI/AAAAAAAAEZ4/lCY0TcBt2uo/s1600/004.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://3.bp.blogspot.com/-8543c5R3Lro/UtxKaCLhblI/AAAAAAAAEZ4/lCY0TcBt2uo/s1600/004.JPG" height="213" width="320" /></a></div><br />We will pick up his new hearing aids on Wednesday. I am looking forward to it, because every other word is "what?" at the moment. I think his old aids are on their last legs!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-47256255255271105912014-01-15T07:09:00.002-08:002014-01-15T07:09:47.125-08:00New Glasses and New Hearing Aids: FAILOn Tuesday, we were supposed to drive up to Williamsville to get Nolan's new hearing aids. The audiology center called and confirmed the appointment, I took the day off work, and sent Nolan to school on Tuesday morning in a state of glee - he was very excited about his new "ears."<br /><br />The phone rang an hour before I was supposed to leave. The audiologist called, and said that the hearing aids didn't have the correct FM receivers, so there was no point in driving up to Williamsville, since we'd just have to return a few days later to have the FM receivers installed.<br /><br />Apparently, the audiologist had noticed the wrong FM receivers were shipped from Phonak when they arrived in December, and had her staff member place an order for the correct ones. The proper receivers never arrived, and she checked on the status the day we were to drive up to collect the hearing aids. Phonak had no record of Nolan's name and no record of the order. The new FM receivers have been ordered and are on a "rush" status, but we won't be able to collect them until Wednesday the 22nd. Nolan was beyond disappointed, but what can you do?<br /><br />On the bright side, at least they managed to call BEFORE we had driven for 2 hours to the hearing center.<br /><br />Today, we were supposed to pick up Nolan's new glasses. I took another day off work, and Nolan missed his morning classwork. We arrived at Spectrum and the technician pulled out a set of frames. Blue and brown ones.<br /><br />Nolan said, "They're supposed to be green!"<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-WO6vZg9kp1Y/Utaja1-_JhI/AAAAAAAAEWM/QmNMyKqbduc/s1600/003.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://3.bp.blogspot.com/-WO6vZg9kp1Y/Utaja1-_JhI/AAAAAAAAEWM/QmNMyKqbduc/s320/003.JPG" width="320" /></a></div><div style="text-align: center;"><i>Nolan with his old glasses at the optometrist. This was supposed to be the "before" picture.</i></div><br />There was some argument - the tech insisted these were the correct frames, until she checked the serial number we had actually ordered - but we had ordered the frames with the green arms to match Nolan's ski helmet. Once the records were checked, she admitted the wrong frames had been ordered.<br /><br />So Nolan doesn't have new glasses, either.<br /><br />Unfreakingbelievable.<br /><br />Now I get to take 2 more days off work - though we're not sure when the new glasses will be in. They have to order new lenses and get the proper frame sent to the store.<br /><br />Hopefully sometime by the end of January, Nolan will have new hearing aids and glasses. Heavens knows that we pay enough for these necessary items - if only the medical staff were competent enough to provide them!<br /><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-8554678851547956642014-01-10T16:07:00.000-08:002014-01-10T16:07:24.728-08:00Happy 6th Hearing BirthdayNolan's sixth hearing birthday was yesterday. I would have posted a blog then.. but, truth be told, I completely forgot. I didn't remember until the audiologist called to confirm his appointment on the 14th, when I suddenly realized - hey, it is January 10th! Yesterday was the anniversary of when he first heard sound!<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-rMGmc5g8LBA/UtCGPuC1yQI/AAAAAAAAEV0/OHoBUylXsec/s1600/035.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://4.bp.blogspot.com/-rMGmc5g8LBA/UtCGPuC1yQI/AAAAAAAAEV0/OHoBUylXsec/s1600/035.JPG" height="320" width="240" /></a></div><br />He has grown a lot since those first moments with his hearing aids. I'll never forget the day before he received his first hearing aids. It was a sunny day in January, and I was so anxious about the future. How would we communicate? Would he lose more hearing? Was anything else wrong with our precious baby? Would he talk? What would his life be like?<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-1Tuji5R68O4/UtCGyPRVn3I/AAAAAAAAEV8/wouQeNlad_4/s1600/IMG_2578.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://1.bp.blogspot.com/-1Tuji5R68O4/UtCGyPRVn3I/AAAAAAAAEV8/wouQeNlad_4/s1600/IMG_2578.JPG" height="213" width="320" /></a></div><br />The next day, his ears were born. He could hear <i>something</i>&nbsp;without hearing aids at this point in his life. He had a moderate hearing loss, but all speech sounded like Charlie Brown's teachers. Hearing aids would bring those blurry sounds into focus - allowing him to hear <b>all</b>&nbsp;the sounds of speech.<br /><br /><iframe allowfullscreen="" frameborder="0" height="315" src="//www.youtube.com/embed/WClxCx3ksBM" width="420"></iframe> <br />My bobble-headed baby boy loved sound, and was a mere four months of age when we turned on his ears. Over time, answers came to my questions.<br /><br />Would he lose more hearing? Yes. Nolan now has a moderately severe to severe hearing loss. He is likely to continue to lose hearing (albeit slowly). With technology, however, this isn't as much of a worry - he will always have access to sound, even if we have to change the technology down the road.<br /><br />As for how we would communicate - well, we often have to tell Nolan to take a five minute break from talking. He is the most verbal person in our family. His receptive and expressive language is off the charts. He talks. He sings. He plays the piano. And sometimes the pipe organ.<br /><br /><iframe allowfullscreen="" frameborder="0" height="315" src="//www.youtube.com/embed/TGQ6LKs2jg0" width="560"></iframe> <br /><br />He is one of the top readers in his class. I used to worry that he would need help with his reading - as it turns out, he is pulled out for a special reading group. This reading group, however, is for the advanced readers in the first grade.<br /><br />Was anything else wrong with him? That question unfolded over the years. For several terrifying years, we agonized over what was going on with our little boy. The inability to grow, breathing difficulties, constant ear and sinus infections, amblyopia... every new symptom caused a wave of panic. Now that we have some answers, we are able to help him and avoid the vomiting attacks and other problems that come with dysautonomia (one of the unfortunate conditions that often comes along with Ehlers Danlos Syndrome). He is growing. His vision is now correcting to 20/30, and we have stopped the sinus and ear infections. Some things aren't "fixable," but he is stable and we know how to help him. I finally feel like we have a handle on things.<br /><br />And as for what his life would be like.. well, it is simply <i>regular.</i>&nbsp;He goes to a regular first grade class. He plays on a soccer team in the summer. He is a bit of a daredevil and rides roller coasters that I will never, ever dare to ride. He calls himself the "green bullet" when he darts down the ski hill. He is a confident, articulate, rambunctious six year old boy.<br /><br />Life is good.dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com1tag:blogger.com,1999:blog-2074245988180152453.post-36738352678380643332014-01-08T12:18:00.003-08:002014-01-08T13:23:30.063-08:00New Hearing Aids and GlassesWe haven't picked them up yet, but Nolan's new hearing aids are on order. We will drive up to Buffalo on January 14th to pick them up - we chose the Bolero Q70 SP model. These hearing aids have 16 channels (as opposed to the eight channels Nolan currently has) and are water-resistant. They also have an integrated FM receiver.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-vKfFoVZBoVo/Us2xTOVW1oI/AAAAAAAAESc/FtDQGYiR0fc/s1600/032.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://2.bp.blogspot.com/-vKfFoVZBoVo/Us2xTOVW1oI/AAAAAAAAESc/FtDQGYiR0fc/s1600/032.JPG" height="213" width="320" /></a></div><br />The integrated FM is a huge plus. Nolan's personal FM system has not been working properly since he started school in September. He has a Phonak Inspiro, and it has had multiple issues. First, the transmitter was sent to Phonak and they did some basic maintenance... but the transmitter would not work once it was sent back to the school. Phonak replaced the transmitter when it was sent in for the second time, as there was a faulty circuit board. Then it was sent in for a third time because it wouldn't work. Now, one hearing aid won't register the FM signal - but this time, we think it is the hearing aid and not the FM system. Hopefully getting Phonak aids (instead of the Unitron aids he currently has) will help solve our FM problems. While his teachers are so happy to state that he "compensates well," lip reading and filling-in-the-blanks is not the same as actual hearing. He misses a lot.<br /><br />He is also getting new glasses, which will be picked up the day after getting his new hearing aids. I am excited for his soon-to-be improved vision and hearing!<br /><br />In other news, the windchill has caused our school to cancel for two days in a row (-30 Fahrenheit with the windchill, -12F as the "regular" temperature). It is warming up tonight, and will actually rain on Saturday. The kids are getting a bit antsy, as the weather has been too cold to play outside and there is a lot of pent-up energy here!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-35034072385542402022014-01-06T15:44:00.001-08:002014-01-06T15:45:06.794-08:00Matt's pH Probe Results<div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-dlEf0Ref64k/Uss9UhtU2mI/AAAAAAAAER4/0b2FfVLJ0M8/s1600/028.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://1.bp.blogspot.com/-dlEf0Ref64k/Uss9UhtU2mI/AAAAAAAAER4/0b2FfVLJ0M8/s1600/028.JPG" height="240" width="320" /></a></div><br />In November, we received a call from our ENT's office with Matthew's pH probe results. The probe shows he has severe nighttime reflux - over 109 episodes while he was asleep. This explains his frequent apneas and sleep arousals, which results in very little REM sleep.<br /><br />Coincidentally, we had noticed a lot of behavior problems starting in October - I didn't make the connection at the time, but we took him off his anti-reflux medication prior to the pH probe. In hindsight, his emotional meltdowns and ADD type behavior was directly caused by the increase in reflux when the medication was removed.<br /><br />The probe was extremely valuable for a number of reasons, including:<br /><br />1. We know he has acid reflux affecting his airway, as opposed to other conditions that can mimic reflux.<br />2. We know he doesn't reflux during the day at all, but has severe reflux when sleeping.<br /><br />The second point is particularly important. Originally, Matthew was on a low-dose (10 mg) of Prilosec, given once per day in the morning. &nbsp;The information gained from the probe gave us the ability to medicate his GERD more appropriately: he is now on 40 mg of Nexium, given in the evening.<br /><br />Once we started the Nexium, the turn-around in his behavior was phenomenal. He is no longer melting down on a regular basis, is able to focus, and is able to reason again.<br /><br />We return to the ENT's office on February 5 to discuss his progress and determine what the next step should be (likely another sleep study to keep tabs on his apnea).<br /><br />I have been absent from this blog - I am now working outside the home part-time and life is insanely busy! It is a whirlwind of work/school/tennis/piano/ski club: all positive, wonderful things, but writing has been placed on the back burner!<br /><br />I will update on Nolan in another post - Happy 2014, everyone!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-64451696390114066742013-10-25T15:57:00.001-07:002013-10-25T15:57:29.469-07:00pH Probe for Matthew<div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-HeJ4RuAzdJo/Umr1Ioy4V6I/AAAAAAAAEMQ/AfC9usCysgU/s1600/007.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://4.bp.blogspot.com/-HeJ4RuAzdJo/Umr1Ioy4V6I/AAAAAAAAEMQ/AfC9usCysgU/s320/007.JPG" width="320" /></a></div><br />We drove up to Buffalo again this morning to have Matt's pH probe placed. His supraglottoplasty failed and he still has sleep apnea, so we need to work out the reasons why he still has poor sleep and respiratory issues during sleep.<br /><br />During his last laryngoscopy, his airway was very red and inflamed. Since he has the laryngomalacia, a presumptive diagnosis of reflux was made and he was placed on Prilosec. A very conservative supraglottoplasty was done (just trimming one floppy side instead of both).<br /><br />Since he still has apnea, we need to accurately determine if he has airway reflux before considering any further steps. Since he has never been formally tested for reflux, we were told to take him off his anti-reflux meds a month ago. This morning, a probe was placed to detect reflux in aerosol form in his upper airway.<br /><br />Placing the probe was, of course, an awful ordeal. No child wants to have a tube placed up their nose and down their throat. I won't go into detail, but let's just say that four nurses were involved, along with a papoose board and a lot of tears. Fortunately, the probe was placed quickly and in one try. Once it was in, Matt was fine.<br /><br />We went home and he's been building a big Lego airplane we bought for him. Thank goodness for Legos.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-G88nUvh8nPk/Umr26-m4KOI/AAAAAAAAEMc/Vdu-9v6yAkw/s1600/006.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://2.bp.blogspot.com/-G88nUvh8nPk/Umr26-m4KOI/AAAAAAAAEMc/Vdu-9v6yAkw/s320/006.JPG" width="320" /></a></div><br />We return in the morning to have it removed. I haven't actually seen any reflux events, so it will be good to determine if he actually has reflux or not. If he doesn't, then he doesn't need to take medication for it. In addition, revision surgery for his airway would be much less risky.<br /><br />We'll see what the data shows.. we will get the results on December 4.dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com4tag:blogger.com,1999:blog-2074245988180152453.post-27617460927729445672013-10-25T12:04:00.001-07:002013-10-25T12:04:29.933-07:00Hearing Tests We took the boys up to Buffalo for hearing evaluations on Tuesday. Nolan didn't have a hearing test, but we did do another aided audiogram (new audiologist) and selected new hearing aids. We are still waiting on a price quote, but Nolan will be getting Phonak Bolero Q hearing aids. We're quite excited about this, as we feel it will let him hear better. Technology has improved over the past 6 years, so new hearing aids are a good thing!<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/--BIAn-NIVmg/Umq9RzWacFI/AAAAAAAAEIU/mlfgBKA0iFc/s1600/001.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/--BIAn-NIVmg/Umq9RzWacFI/AAAAAAAAEIU/mlfgBKA0iFc/s320/001.JPG" width="320" /></a></div><br />Nolan did get new impressions, as he will receive new earmolds with the new hearing aids. He chose a new color, too - he loves changing his earmold colors!<br /><br />Matthew had testing for central auditory processing disorder, which is called CAPD, CAP, or APD, depending on where you live. He does NOT have APD, which is good to rule out, as it has a similar symptom set as ADD and as sleep apnea. We already know Matt has sleep apnea, so the inattention/moodiness/zoning out are due to the apnea and nothing else.<br /><br />We did, however, find that Matthew has developed some very minor hearing loss. It is nothing that can or should be aided at this point, but his audiogram looks something like this:<br /><br />He used to test as 15dB across the board (normal for children). While the main area of the speech banana is not affected, he is now on a yearly monitoring schedule so that we can aid him if and when the loss enters the speech range.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-CGLdrmFhdBc/Umq_ssUF3QI/AAAAAAAAEIc/hb4PaYteTKo/s1600/mattaudiogramoctober2013.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="262" src="http://1.bp.blogspot.com/-CGLdrmFhdBc/Umq_ssUF3QI/AAAAAAAAEIc/hb4PaYteTKo/s320/mattaudiogramoctober2013.jpg" width="320" /></a></div><br />I am waiting to receive his audiogram in the mail, as this might be slightly "off" from the actual one. The right ear is slightly better than the left ear. Basically, he drops to 50dB at 8,000 Hz (ultra high frequencies) and touches mild in the low frequencies. He is "normal" for an adult hearing level, but has a slight hearing loss for the pediatric age range (better than 15dB hearing level). This does explain why he mishears the S vs. SH sounds. He can 'hear,' but might have difficulty in noise and might have some trouble with ultra high frequency sounds.<br /><br />This does explain some of the "ADD" type behaviors.. calling his name when he's watching TV and in a busy location - it is not the busy nature of the situation that is distracting him.. he is having difficulty hearing with clarity in noisy situations. In any case, this is something to keep a close eye on.<br /><br /><br /><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-9123267708607293072013-10-21T11:10:00.001-07:002013-10-21T11:10:20.414-07:00October Update<div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-tfxLLcPJnDk/UmVqeyJV6oI/AAAAAAAAEAg/Tknf1nfY8Yg/s1600/042.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://1.bp.blogspot.com/-tfxLLcPJnDk/UmVqeyJV6oI/AAAAAAAAEAg/Tknf1nfY8Yg/s320/042.JPG" width="320" /></a></div><br />Wait, that's right... I have a blog! Perhaps I should update it...<br /><br />I have several very good excuses for not writing:<br /><br />1) The kids returned to school and our routine activities keep us busy.<br />2) I have returned to work part-time and now barely have time to breathe.<br />3) It looks like this outside:<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-QaIRs_gJoyw/UmVrIAd4XQI/AAAAAAAAEAo/J41pkS55iTE/s1600/020.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://1.bp.blogspot.com/-QaIRs_gJoyw/UmVrIAd4XQI/AAAAAAAAEAo/J41pkS55iTE/s320/020.JPG" width="320" /></a></div><br />When it is beautiful outside, I relish every last second of it. Snow is due to hit this Wednesday, so we have been outside soaking up the vibrant beauty of fall.<br /><br />Nolan's ENT has not been able to return to work. I &nbsp;heard a very devastating rumor, but have not been able to confirm it. In the meantime, we are praying for her and for her family. We have been moved to another ENT in the same practice, who is now managing both boys' care.<br /><br />Nolan is fairly stable at the moment. He did have another ear infection in September, but a round of Ciprodex cleared it up fairly quickly. He did have to start school with one ear, but managed to compensate well for the lack of hearing. We do want to look into new hearing aids for him and to obtain an aided audiogram/real-ear testing on a regular basis. He has a hearing aid evaluation tomorrow afternoon - we would like to get water resistant aids with updated technology.<br /><br />Matthew failed his last sleep study, so his appointment was a bit longer. The new ENT was reviewing his records and decided that we should perform a pH probe to determine if his red/burned airway was truly due to reflux - or some other reaction (there are conditions that mimic reflux). Matt will have his pH probe placed on Friday and will have it taken out on Saturday.<br /><br />In addition, we aren't certain if some of his ADD type behaviors are due to sleep apnea or to other factors. The new ENT would like to run testing for central auditory processing disorder, which is often confused with ADD (inattentive type). The testing for this disorder is very similar to a hearing test, so he will have testing performed at the same time as Nolan. Two birds, one stone.. at least we're efficient with our appointments.<br /><br />This means I have three trips to Buffalo this week, and an oral surgeon appointment (I broke a tooth and will have it removed/probably get an implant). Did I mention I am working part time? Some weeks it is <i>very</i>&nbsp;part time. Sigh...<br /><br />I will try to update more frequently - I keep waiting for things to slow down, but that doesn't appear to ever happen!<br /><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com1tag:blogger.com,1999:blog-2074245988180152453.post-57816055867649221782013-09-12T15:15:00.000-07:002013-09-12T15:15:28.335-07:00Pediatric Rheumatologist Appointment<div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-kufHLYepfUM/UjI0moQ4R4I/AAAAAAAAEAM/DdCvJiDKPLM/s1600/002.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://2.bp.blogspot.com/-kufHLYepfUM/UjI0moQ4R4I/AAAAAAAAEAM/DdCvJiDKPLM/s320/002.JPG" width="320" /></a></div><br />Goodness gracious, it has been hot and <i>humid</i>&nbsp;lately. It was 95 degrees with 98% humidity the other day, and we were miserable in the sultry heat. Living in Western NY, we don't have central air conditioning, because summer typically lasts approximately 5 minutes and winter lasts for the rest of the year. Nolan sweats more than most kids, so he was literally soaked through in this weather.<br /><br />Three days ago, a dear friend called to tell me that our beloved ENT was canceling all of her appointments. More specifically, her office was canceling the appointments on her behalf. There has been some sort of catastrophe and they do not know if she will be seeing patients again. I still have no idea what has happened, but I am worried for her and for her family. Of course, this leaves us in a bit of a lurch, as Matthew still has significant sleep apnea after his supraglottoplasty. Nolan is fairly complex as an ENT patient as well, so we need to find a replacement that can handle the boys' issues. I have a call into our ENT's nurse practitioner and we will see what she recommends.<br /><br />Since Nolan had a pediatric rheumatology appointment, I thought I'd ask her opinion, too. His rheumatologist is fantastic, and always takes a good deal of time with our family.<br /><br />We talked about our latest concerns, which include:<br /><ul><li>Bradycardia (slow heart rate) when sleeping - his heart rate dips into the 30's on some occasions.</li><li>Difficulty with sustained walking.</li><li>Hearing loss and management.</li><li>Periodic leg movements and waking at night.</li></ul><div>Nolan's weight is hanging in there at 42 pounds, and he is 45.5 inches tall. His nighttime feeds are going well, so that part of his care is fine.</div><div><br /></div><div>She wants Nolan to see a cardiologist, because his last echocardiogram and EKG were when he was 3 years old, before he had developed the heart murmur he has now. Adding in the bradycardia, it would be good to get a consultation. It is likely that everything will be reported as fine, but kids with connective tissue disorders have a higher risk of having cardiac issues. Better safe than sorry, in any case.</div><div><br /></div><div>As far as his hearing and other ENT issues, she has a few recommendations. She is going to contact a few other families in the area to see what they are doing with regard to seeing an ENT.&nbsp;</div><div><br /></div><div>The periodic leg movements are not due to electrolyte deficiencies or to anemia (he had had blood work during the same time period as his sleep study), so it is likely there is a muscular or nervous system issue at work there. He has some minor central apnea as well as the gastroparesis, so there is something "going on" with his autonomic system. They can treat leg movements with amitriptyline, but this relaxes the palate and airways... which makes the medication a bad idea for a child with congenitally floppy airways. We'll just ignore the problem for now - there isn't much we can do about it. I am driving him to school, which lets him sleep a bit longer in the mornings.</div><div><br /></div><div>Nolan's lack of endurance and fatigue is becoming a serious issue. Currently, we are carrying him when we are in a city or on vacation. As he grows, carrying him is becoming more difficult. He doesn't fit into a standard umbrella stroller anymore, so the time has come to look into a medical stroller. He won't need to be fitted for a pediatric wheelchair, as he doesn't need full-time use - he just needs a mode of transportation when his legs and body give out.&nbsp;</div><div><br /></div><div>We're going to look for one second-hand (Maclaren makes a medical stroller called the "Major," which isn't horrifically expensive - less than $1,000). With Nolan's complexity and mounting medical costs, she wants to investigate acquiring Medicaid as a back-up for covering medical costs. Most medically complex children have Medicaid, because private insurance does not cover DME - in the past, we have paid for Nolan's g-tubes, C-pap, and hearing aids out of pocket. Now we're adding a medical stroller. If he qualifies, we'd use our private insurance for most things, but the Medicaid for the surprising number of things private insurance doesn't cover. His medical costs can be staggering at times, so having a little help for things our insurance company won't cover.</div><div><br /></div><div>It was a very productive appointment, and she gave us a few good ideas on where to look for a second-hand medical stroller.</div><div><br /></div><div>We return to Buffalo on Saturday for Nolan's MRI (inner ear).&nbsp;</div>dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com3tag:blogger.com,1999:blog-2074245988180152453.post-17010140937930147312013-09-12T14:38:00.001-07:002013-09-12T14:38:28.673-07:00Ear Infection (Again)<div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-No1Oz2zSAJc/UjIy6Mm-yYI/AAAAAAAAD_4/804tiS5Palk/s1600/001.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://3.bp.blogspot.com/-No1Oz2zSAJc/UjIy6Mm-yYI/AAAAAAAAD_4/804tiS5Palk/s320/001.JPG" width="320" /></a></div><br />Nolan had a fantastic time the week of his birthday. He went tubing on the lake, went to a water park, and we took a trip to Toronto.<br /><br />Unfortunately, all the heat/humidity/water activities &nbsp;resulted in a raging ear infection in his right ear. I was able to acquire more Ciprodex drops and started treating it quickly, but Nolan had to start first grade with only one ear.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-T81_6-1dpU8/UjIzj-P1UBI/AAAAAAAAEAA/h3ozTguey3o/s1600/002.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://1.bp.blogspot.com/-T81_6-1dpU8/UjIzj-P1UBI/AAAAAAAAEAA/h3ozTguey3o/s320/002.JPG" width="213" /></a></div><br />Thankfully, his new teacher of the deaf (TOD) is beyond fantastic. She actually trained at the Clarke School (an auditory-verbal program) and is thrilled to see Nolan is reading at an advanced level. He is having significant problems hearing, so his desk has been moved to the front of the teacher's desk, and he has daily pull-outs to work on the curriculum in a 1:1 environment. He is adjusting to first grade well, and loves his teacher(s).<br /><br />His ear took about a week to clear up. Hopefully we won't see another ear infection for a while!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com0tag:blogger.com,1999:blog-2074245988180152453.post-10123515270483071982013-08-31T18:23:00.000-07:002013-08-31T18:23:06.527-07:00Matt's Sleep Study Results (Post Supraglottoplasty)<div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-z3Bq1EG2gjk/UiKVM27Ye4I/AAAAAAAAD-Q/MxmeD2Fhkio/s1600/077.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://1.bp.blogspot.com/-z3Bq1EG2gjk/UiKVM27Ye4I/AAAAAAAAD-Q/MxmeD2Fhkio/s320/077.JPG" width="320" /></a></div><br />Matt's follow-up appointment wasn't until October 28 with the ENT - I figured if there was any "negative" news, we'd find out soon enough.<br /><br />The ENT's office called and said his apnea is unchanged despite doing a tonsillectomy and adenoidectomy and a supraglottoplasty. They re-booked his appointment for September 22 to re-evaluate his case.<br /><br />I don't have an official report yet, but the short summary is this:<br /><br />The bad news: Matt still has moderate/severe sleep apnea.<br />The good news: I didn't throw out the C-Pap machine!<br /><br />At least Nolan's apnea is under control for the time being, so we can focus on getting Matt's under control.<br /><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com1tag:blogger.com,1999:blog-2074245988180152453.post-82818405418076470292013-08-11T15:23:00.000-07:002013-08-11T15:23:01.462-07:00Matt's Sleep Study: Post-Supraglottoplasty<div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-oe_I3mrwOMc/UggMCSLd0vI/AAAAAAAAD9c/rOB_4YJRs7U/s1600/mattsleepstudy2.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://2.bp.blogspot.com/-oe_I3mrwOMc/UggMCSLd0vI/AAAAAAAAD9c/rOB_4YJRs7U/s320/mattsleepstudy2.jpg" width="320" /></a></div><br />I was happy to get a sleep study booked for Friday night - this meant that I would be able to sleep on Saturday during the day, since Dennis would be home to watch the boys. I drove Matthew up to Buffalo Children's and we were ushered into the waiting room just as the sun was starting to set.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-i6wIsJLNbJ0/UggMv3xX94I/AAAAAAAAD9k/AeUIkIkH5aM/s1600/buffalochildrenssunset.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://3.bp.blogspot.com/-i6wIsJLNbJ0/UggMv3xX94I/AAAAAAAAD9k/AeUIkIkH5aM/s320/buffalochildrenssunset.jpg" width="320" /></a></div><br />The sleep study tech was not quite ready for us, so she sent us to the playroom. Matthew was happy to play with the toys. I, however, was <i>not</i>&nbsp;happy that someone had changed the channel on the waiting room television to play Bones. This television show is definitely not for children, and Matthew was rapt with attention as they showed a half-rotted corpse on the show. I finally found a power button on the side of the TV. Matt went back to playing with his pirates. I was not happy.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-Cvaqz8q4SDs/UggNOnU4_XI/AAAAAAAAD9w/uqoD3Ier7Lk/s1600/mattplaywithtoys.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://3.bp.blogspot.com/-Cvaqz8q4SDs/UggNOnU4_XI/AAAAAAAAD9w/uqoD3Ier7Lk/s320/mattplaywithtoys.jpg" width="320" /></a></div><br />We were finally ushered into a room and Matt changed into his pajamas. Matt played on the Kindle Fire with the Bad Piggies game, using it to test out several rocket designs. Sleep studies are <i>much</i>&nbsp;easier at this age, as the kiddos can entertain themselves!<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-yN7q3QDUP_w/UggNlwjq9yI/AAAAAAAAD94/cOYI_0qUUiE/s1600/mattplaytablet.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/-yN7q3QDUP_w/UggNlwjq9yI/AAAAAAAAD94/cOYI_0qUUiE/s320/mattplaytablet.jpg" width="320" /></a></div><br /><br />At 9:30, hook-up started. By 10:00, Matt was settled in bed and I was reading a chapter of Harry Potter and the Sorcerer's Stone. He wasn't happy about the nasal cannula, but left it alone. He's actually giggling in the picture below - I was worried it would take him a long time to fall asleep.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-micn_0XsJB0/UggN5fA833I/AAAAAAAAD-A/c9O6flXTozU/s1600/mattsleepstudygiggle.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://3.bp.blogspot.com/-micn_0XsJB0/UggN5fA833I/AAAAAAAAD-A/c9O6flXTozU/s320/mattsleepstudygiggle.jpg" width="320" /></a></div><br />Fortunately, he fell asleep almost as soon as I turned the lights out. He stayed asleep until 2:30 am, when he woke briefly and said, "I think it is dusk outside," then immediately fell back to sleep. I managed to get about an hour of sleep, which is <i>much</i>&nbsp;better than getting no sleep at all. At least I would be somewhat coherent on my drive back to Jamestown.<br /><br />The lights came on at 5:00 am, and we grabbed some Tim Horton's donuts (and a coffee for me). We were home by 7:30 am. I thought I would nap during the day, but I have a hard time sleeping during daytime hours. Matt and I both had early bedtimes that night, though!<br /><br />I won't get the results from Matthew's sleep study until October 28, which is when we have our follow-up appointment with the ENT. Hopefully his sleep apnea is gone!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com3tag:blogger.com,1999:blog-2074245988180152453.post-32000526306197002892013-08-07T04:27:00.000-07:002013-08-07T04:27:57.602-07:00Nolan's Sleep Study Results<div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-nGFMjwL_Ztg/UgItcPTYIDI/AAAAAAAAD4o/rRKpYsxegjY/s1600/018.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://1.bp.blogspot.com/-nGFMjwL_Ztg/UgItcPTYIDI/AAAAAAAAD4o/rRKpYsxegjY/s320/018.JPG" width="320" /></a></div><br />Nolan's sleep study was an exhausting, sleepless ordeal in late June. He woke frequently and cried. His legs bothered him a lot, and it was obvious from the sleep study report that he has difficulty with periodic leg movements and frequent arousals. He also has moments of bradycardia where his heart rate drops well below 40 beats per minute - this was observed in the PICU when he had his supraglottoplasty as well. He is fine during the day, so it is probably fine - but we will bring it up with his pediatric rheumatologist when we see her in September (along with the leg issues). Dysautonomia occurs with children who have Ehlers Danlos, so it is possible he isn't regulating his heart rate well. Better safe than sorry where the heart is concerned!<br /><br />On the other hand, we received some fantastic, out-of-this-world news. His second supraglottoplasty/epiglottopexy actually <i>worked</i>. His apnea is now in the mild range for the pediatric age group. This means that we do not have to use C-Pap for the time being. He will have another sleep study next year, as he is high risk for the apnea to return with his connective tissue disorder. We are absolutely <i>thrilled</i>&nbsp;that we do not have to cart around a C-Pap machine and he is getting the oxygen he needs to grow.<br /><br />So, goodbye C-Pap machine. We won't miss you.<br /><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com6tag:blogger.com,1999:blog-2074245988180152453.post-48966474677443399752013-08-06T06:48:00.001-07:002013-08-06T06:50:16.717-07:00Still Here - Playing Catch-Up and Matt's SupraglottoplastyI have been absent from the bloggy world. I have a good excuse. We had four - count them, <i>four</i>&nbsp;- surgeries this past spring. Two for Matthew and two for Nolan.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-MJMoParoEBk/UgD5saw6IuI/AAAAAAAAD3g/NGnsfOK6WEI/s1600/nolanhospital.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/-MJMoParoEBk/UgD5saw6IuI/AAAAAAAAD3g/NGnsfOK6WEI/s320/nolanhospital.jpg" width="320" /></a></div><br />First was Nolan's attempted supraglottoplasty in February, which couldn't be completed because he had funny, white exudate all throughout his airway. He had to be woken up from general anesthesia and we were sent home. The cultures were normal, so this mystery was never explained.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-JNaqN52_Wyg/UgD6QEVogMI/AAAAAAAAD3o/604OUfRVa0c/s1600/019.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://4.bp.blogspot.com/-JNaqN52_Wyg/UgD6QEVogMI/AAAAAAAAD3o/604OUfRVa0c/s320/019.JPG" width="320" /></a></div><br />Second was Matt's tonsillectomy and adenoidectomy in March. An outpatient surgery with 8 long days of recovery.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-b9rl8Rgnwqc/UgD6t-DAVEI/AAAAAAAAD3w/trIMFa3hTXY/s1600/018.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/-b9rl8Rgnwqc/UgD6t-DAVEI/AAAAAAAAD3w/trIMFa3hTXY/s320/018.JPG" width="320" /></a></div><br />Third was Nolan's supraglottoplasty and 3 day stay in the PICU in April. He was intubated on a ventilator for 2 days.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-KpjHDB4DXjM/UgD7BjwLxkI/AAAAAAAAD34/cLmRDOJ5Jxk/s1600/MattandBlaze.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/-KpjHDB4DXjM/UgD7BjwLxkI/AAAAAAAAD34/cLmRDOJ5Jxk/s320/MattandBlaze.jpg" width="320" /></a></div><br />Fourth was Matthew's supraglottoplasty and overnight stay in the PICU for observation on June 25, 2013.<br /><br />Honestly, I plead exhaustion. It was simply a ridiculously exhausting spring.<br /><br />Matthew's surgery went <i>very</i>&nbsp;well. Nolan's genetic syndrome (Ehlers Danlos) wreaks havoc on his tiny body, and it is much more evident when comparing the boys' surgery recoveries. Nolan was in a lot of pain and had a lot of trouble swallowing and required ventilation assistance after surgery. Matthew cried for an hour after surgery, then demanded French toast and a cheeseburger.<br /><br />Unfortunately, he was only allowed clear liquids for the rest of the day. The morning, however, brought French toast and an ecstatic Matt. "Mommy, this is the goodest breakfast <i>ever</i>!"<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-dbC8uj4X2ks/UgD8sW3hAuI/AAAAAAAAD4I/WZl62RGykFo/s1600/hungrymatt.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://2.bp.blogspot.com/-dbC8uj4X2ks/UgD8sW3hAuI/AAAAAAAAD4I/WZl62RGykFo/s320/hungrymatt.jpg" width="320" /></a></div><br />Matt's recovery was ridiculously easy. He didn't have to take any pain medication after the first day in the hospital. We went home 24 hours after the operation. 48 hours after his surgery, he was playing in his baseball team's championship game. They got second place.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-pFL57M1FA9s/UgD9qwICKeI/AAAAAAAAD4Y/6iCAHa3A86A/s1600/090.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://2.bp.blogspot.com/-pFL57M1FA9s/UgD9qwICKeI/AAAAAAAAD4Y/6iCAHa3A86A/s320/090.JPG" width="213" /></a></div><br />Seriously. This is Matthew 48 hours after surgery. His recovery was insanely easy. It was nice to have things go so smoothly!<br /><br />We have a sleep study on August 9, 2013 (this coming Friday) to determine if he still has sleep apnea. I certainly hope his apnea is gone!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com4tag:blogger.com,1999:blog-2074245988180152453.post-57298352735948411722013-06-24T11:32:00.000-07:002013-06-24T11:32:05.716-07:00Sleep Study Number Six<div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-VnAWo9mp6hA/UciMRgNF2HI/AAAAAAAAD2U/jEc63yolui4/s1600/monitor.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://4.bp.blogspot.com/-VnAWo9mp6hA/UciMRgNF2HI/AAAAAAAAD2U/jEc63yolui4/s320/monitor.jpg" width="320" /></a></div><br />Nolan had his follow-up sleep study from his revision supraglottoplasty last night. I am absolutely exhausted, so this post may be a complete jumble of incoherent mumbling. Fair warning.<br /><br />We arrived at Buffalo Children's for the hook-up at 8:00 pm. The children's hospital is much better than the alternate sites because the tech is familiar with children and all of the equipment is properly sized. They also perform CO<span style="font-size: xx-small;">2</span> monitoring at the children's hospital and do not perform this test at the alternate sites.<br /><br />Nolan was a champ with the hook-up. He's so used to the process that he didn't blink an eye.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-O_WqCnoH5tg/UciNZCilGCI/AAAAAAAAD2k/Obe7olntbxU/s1600/hookup.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://4.bp.blogspot.com/-O_WqCnoH5tg/UciNZCilGCI/AAAAAAAAD2k/Obe7olntbxU/s320/hookup.jpg" width="320" /></a></div><br />The only part of the process that he hates is the nasal cannula. He couldn't remember what it was called and kept telling us that he "hated that mustache!" He turned the volume up to maximum on the TV speaker and watched some Sesame Street.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-EnfUlL4n078/UciN-Pdn-5I/AAAAAAAAD2s/eD_nAqcbfwA/s1600/tvwatching.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/-EnfUlL4n078/UciN-Pdn-5I/AAAAAAAAD2s/eD_nAqcbfwA/s320/tvwatching.jpg" width="320" /></a></div><br />The same episode plays in a loop, so our wonderful respiratory technician brought in a DVD player with Toy Story. Nolan was quite happy with the arrangement, particularly when she rearranged his head wrap so he could put his hearing aids back in.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-yG1z4Sa_7uw/UciOpPB3U3I/AAAAAAAAD20/vNEaInClVJk/s1600/toystory.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://1.bp.blogspot.com/-yG1z4Sa_7uw/UciOpPB3U3I/AAAAAAAAD20/vNEaInClVJk/s320/toystory.jpg" width="320" /></a></div><br />He fussed a little when the movie was over, and asked us to take his "mustache" off several times. He finally fell asleep, but not for long, He cried out frequently, but the tech came in and told me it was during stage 3 sleep. His legs moved all the time, waking him from sleep. I heard a few apneas, but I am not sure if they were central (some centrals are normal) or if they were obstructive apneas.<br /><br />With the constant thrashing and crying out, I did not sleep at all. At 5:00 am, the lights came on.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-ui2ApVZLuig/UciPTcQK7xI/AAAAAAAAD28/4a9MDSCC-Jc/s1600/sleepstudylightson.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://4.bp.blogspot.com/-ui2ApVZLuig/UciPTcQK7xI/AAAAAAAAD28/4a9MDSCC-Jc/s320/sleepstudylightson.jpg" width="320" /></a></div><br />We woke Nolan up. Well, we sort of woke him up. He was in a zombie-state for quite some time.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-FhqBzNXVuoE/UciPlwtbd-I/AAAAAAAAD3E/SFH9xWZArVM/s1600/zombieboy.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="240" src="http://4.bp.blogspot.com/-FhqBzNXVuoE/UciPlwtbd-I/AAAAAAAAD3E/SFH9xWZArVM/s320/zombieboy.jpg" width="320" /></a></div><br />We left the hospital and drove 2 hours to our house. Nolan fell asleep on the way home, so he got a little more sleep in the car. I am beyond exhausted.<br /><br />Tomorrow, we get up at 4:00 am to take Matt to the hospital for his supraglottoplasty.<br /><br />At least we're never bored.dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-8455218284620590182013-06-13T15:19:00.001-07:002013-06-13T15:19:42.980-07:00Ehlers Danlos Support Groups and ResourcesThis post is mostly for my own benefit, as I have been searching for EDS support groups online. I just want to link to them here, so I don't lose them!<br /><br />I found one group for parents of children with Ehlers-Danlos Syndrome:<br /><br /><b><a href="https://www.facebook.com/groups/250603065082550/">Ehlers-Danlos Syndrome: Parent Group</a></b><br /><br />There is another Facebook Group for the <a href="https://www.facebook.com/pages/EHLERS-DANLOS-NATIONAL-FOUNDATION/294028895335?ref=ts&amp;fref=ts">Ehlers-Danlos National Foundation (EDNF)</a>, and the <a href="http://www.ednf.org/">main website</a> for the organization.<br /><br />I also found an interesting article on the genetics behind Ehlers-Danlos Syndrome - The European Journal of Human Genetics has the article <b><a href="http://www.nature.com/ejhg/journal/v21/n1/full/ejhg2012162a.html">here</a></b>.<br /><br />I still can't find much in the way of severe hearing loss and its relation to EDS. It isn't a common issue to have with the syndrome - it is possible, but very rare.<br /><br />I have been trying to find cases of EDS similar to Nolan, and have found a few cases that have his gastrointestinal problems. The gastroparesis and cyclic vomiting are Nolan's biggest hurdles in the GI world at the moment, though he is gaining weight and growing with his night feeds.<br /><br />We will have to make some decisions about Nolan's participation in certain sports as he gets older. For now, we are letting him continue with soccer since he 1) loves it and 2) the sport is gentle in the younger leagues. In the U-6 league, there is a lot of chasing the ball and not a lot of contact between players. He will start swimming lessons on July 8, and hopefully he will take to it like he did last year. Swimming is a good activity for kids with EDS.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://1.bp.blogspot.com/-iBOsKmOUoAA/UbpE85keUcI/AAAAAAAAD2E/jNe4q3b9m-0/s1600/042.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://1.bp.blogspot.com/-iBOsKmOUoAA/UbpE85keUcI/AAAAAAAAD2E/jNe4q3b9m-0/s320/042.JPG" width="320" /></a></div><br />With that said, we are heading out in the disgusting drizzle for Nolan's soccer practice. We are not sure where summer is, but we'd like the sun to return to Western NY!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com0tag:blogger.com,1999:blog-2074245988180152453.post-58133710934143303862013-06-12T09:01:00.000-07:002013-06-12T09:01:05.947-07:00New Ear Molds<div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-iKSiQGwyzlE/UbiaPISyzfI/AAAAAAAAD1o/VFn6fbRTGQ4/s1600/026.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://3.bp.blogspot.com/-iKSiQGwyzlE/UbiaPISyzfI/AAAAAAAAD1o/VFn6fbRTGQ4/s320/026.JPG" width="320" /></a></div><br />Nolan was in desperate need of new ear molds. We picked them up last week - he went for the green-and-blue look this time.<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-ZbJm7ashTH4/UbiaiW4gRSI/AAAAAAAAD1w/VSQ1uKsc2nI/s1600/011.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://2.bp.blogspot.com/-ZbJm7ashTH4/UbiaiW4gRSI/AAAAAAAAD1w/VSQ1uKsc2nI/s320/011.JPG" width="320" /></a></div><br />Next week is the last week of school, and we are eagerly counting down the days! Both boys have their class field trips on Friday (Nolan is going to a local playground with his class, and Matthew is going to the Erie Zoo).<br /><br />I have to admit, I am looking forward to summer. It will be nice to get a break from the early morning routine!dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com2tag:blogger.com,1999:blog-2074245988180152453.post-69409549710066109142013-06-11T09:58:00.002-07:002013-06-11T09:58:59.072-07:00Matt's Surgery Scheduled<div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-PqPluBLd4qM/UbdVJmEeogI/AAAAAAAAD1Y/XG01wJqbwHc/s1600/001.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://4.bp.blogspot.com/-PqPluBLd4qM/UbdVJmEeogI/AAAAAAAAD1Y/XG01wJqbwHc/s320/001.JPG" width="320" /></a></div><br />We have been so busy that I haven't had time to write. Baseball, soccer, doctor appointments, school events and projects, and piano recitals have kept us overwhelmed with activities! June is a crazy month.<br /><br />Matt had his ENT follow-up for his tonsillectomy and adenoidectomy (T&amp;A) surgery. He still has very obvious signs of sleep apnea. Since our ENT did the scope at the time of his T&amp;A and found the laryngomalacia, we know that the obstruction is not going to go away without help.<br /><br />Matt received a once-over and he is <i>not</i>&nbsp;hyperflexible like Nolan is, so he probably does not have Ehlers Danlos Syndrome. His reflux, laryngomalacia, and constipation issues are likely coincidental. This is good news, as he should heal well and not have a recurrence of the laryngomalacia once surgery is complete. He will remain on anti-reflux medication indefinitely. <br /><br />Matt's supraglottoplasty has been scheduled for June 25. They will laser out the floppy arytenoid folds so that he can breathe freely at night (if you have no idea what a supraglottoplasty or laryngomalacia are, then read more about it <b><a href="http://leahlefler.hubpages.com/hub/Laryngomalacia-in-Infants-and-Children">here</a></b> and <b><a href="http://leahlefler.hubpages.com/hub/Supraglottoplasty-Surgery-for-Laryngomalacia-What-to-Expect">here</a></b>).<br /><br />Fortunately, my mom is flying out to care for Nolan while we are in the hospital with Matt. We need someone who can take care of his g-tube and give him Zofran (if needed) while we aren't at home. I am grateful for the help, as our only other option is to have one parent at the hospital and one at home with Nolan.<br /><br />Nolan also has new earmolds, but I'll share those in another post.<br /><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com3tag:blogger.com,1999:blog-2074245988180152453.post-68320794057252500582013-05-30T15:44:00.001-07:002013-05-30T15:51:10.860-07:00Finally: A Name - Nolan has a Diagnosis<div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-5toQMaHkI6g/UafPt6GcW9I/AAAAAAAAD0w/7McBgGvS7iQ/s1600/012.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://3.bp.blogspot.com/-5toQMaHkI6g/UafPt6GcW9I/AAAAAAAAD0w/7McBgGvS7iQ/s320/012.JPG" width="320" /></a></div><br />Nolan is happy and playing again, much to my relief. He had a very tough weekend, which involved extreme bloating, an inability to eat, and vomiting. By Saturday he was so weak, he slept most of the day. Zofran did stop the vomiting for a period of time, thank goodness. I was so grateful to have the prescription!<br /><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-mykkhI3dSlQ/UafQZAtnW0I/AAAAAAAAD04/Rg91byjj3po/s1600/002.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://3.bp.blogspot.com/-mykkhI3dSlQ/UafQZAtnW0I/AAAAAAAAD04/Rg91byjj3po/s320/002.JPG" width="320" /></a></div><br />We had his blood tests drawn on Thursday of last week, and I was grateful to get that part of the process done and over with. On Friday, the hospital lab called and stated they didn't get enough blood. Nolan was very brave and didn't cry when he had to get another poke for that last tube of blood.<br /><br />I took him to the rheumatologist on Wednesday. She went over his blood work, which was mostly normal (some mild elevations or low levels, but basically normal). His AST level, which is often elevated, was normal for this round of testing.<br /><br />They tested him for mutations in his MTHFR gene. What is the MTHFR gene, you may ask? The long name is&nbsp;<span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">methylenetetrahydrofolate reductase, because you totally wanted to know that information. In any case, there were two mutations tested. Nolan has a mutation known as A1298C (two copies of the aberrant gene). He does <i>not</i>&nbsp;have the other gene (C677T). People who have both mutant genes often have many health problems, but since Nolan only has one "off" gene, he is likely unaffected by the mutation. It is interesting information to have, but not particularly enlightening for his current subset of symptoms. If you are interested, you can read more about this particular mutation <a href="http://www.mthfra1298c.com/">here</a></span>.<br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">The rheumatologist tested his joints and noted his soft skin and bruises on his legs. His elbows hyperextend. His knees, ankles, toes, fingers, hips, and shoulders were assessed... basically, if Nolan has a joint, it bends more than it should.&nbsp;</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Taking his symptoms together, a picture forms.&nbsp;</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Hyperextensible joints</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Obstructive sleep apnea</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Laryngomalacia (floppy airway)</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Gastroparesis</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Acid reflux</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Hearing loss&nbsp;</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Heart murmur (mitral valve)</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">"Clumsiness"</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Severe astigmatism</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Myopia</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Soft, velvety skin</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">Bruising</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><b>Nolan has been diagnosed with the Classic form of Ehlers-Danlos Syndrome (EDS).&nbsp;</b></span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">The only symptom not covered by this diagnosis is his posterior urethral valves, which are likely not connected to his other problems (he was hit by lightening twice).</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">His hearing loss level is not typical for EDS. The type of hearing loss associated with EDS is often conductive and mild-moderate. Nolan's hearing loss is mixed in the low frequencies and sensorineural in the high frequencies - and the level is moderately severe to severe. His progressive hearing loss is unusual for EDS.</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">EDS is a connective tissue disorder - Nolan's body doesn't have properly formed collagen. Every tissue that contains collagen will be fragile and weaker than it should be. His stomach doesn't contract as it should because the tissue is weak. His eyes have an odd shape because the eyes rely on collagen to keep their shape.&nbsp;</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;">He is already developing arthritis in his ankles.</span><br /><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span><br /><div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-LnBxUwzc8lg/UafT-Fr9edI/AAAAAAAAD1I/VKKJMrvEmx4/s1600/018.JPG" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="213" src="http://3.bp.blogspot.com/-LnBxUwzc8lg/UafT-Fr9edI/AAAAAAAAD1I/VKKJMrvEmx4/s320/018.JPG" width="320" /></a></div><span style="background-color: white; font-family: 'Lucida Grande', LucidaGrande, Lucida, Helvetica, Arial, sans-serif; font-size: 14px;"><br /></span>He fatigues easily because of his loose joints. Since the blood vessels are also made out of collagen, blood has a tendency to pool in Nolan's legs as he walks. This can cause dizziness and fatigue (on top of the fatigue caused by the loose joints). He can't walk as far, or as fast, as a typical child his age. If we are going somewhere that requires a lot of walking, he will require mobility assistance.<br /><br />His rheumatologist is going to get his brain MRI's to look for chiari malformation again. She wants to make sure they ruled it out, since children with EDS often have chiari malformations and can have a specific type of hydrocephalus.<br /><br />In the meantime, we are treating his symptoms as they arise. There is no cure for EDS, so we can only help him symptomatically. Having a diagnosis means everything, despite the lack of a "cure:"<br /><br /><br /><ul><li>Future airway surgeries are likely to be unsuccessful with this disorder. The tissues around his larynx are likely to continue to prolapse. With a diagnosis, we can avoid unnecessary surgery.</li><li>We know that Nolan's inability to walk and leg pain is not a behavioral problem or "growing pains," and that he is in significant pain and must receive assistance when walking.</li><li>He must avoid contact sports to prevent permanent damage to his joints.</li><li>His heart must be monitored. Some people with EDS are prone to aortic root dilation and most develop mitral valve prolapse. Nolan has a heart murmur in the location of his mitral valve, though an echocardiogram was "normal" when he was three. In light of this diagnosis, it is likely that he will have mitral valve prolapse.</li><li>He needs to keep his hydration levels up to help prevent fatigue. His doctor wrote a note to keep gatorade at his desk during school hours.</li><li>We know his gastroparesis is unlikely to improve.</li><li>We are to give Zofran the minute we think he <i>might</i>&nbsp;start vomiting, rather than waiting for the vomiting attack to begin.</li></ul><div>In any case, we are glad to have a diagnosis for the little guy. We can be proactive to keep him as healthy as possible and try to limit the damage to his joints. I do wish we had this diagnosis prior to attempting the second supraglottoplasty, though!</div><br /><br />dleflerhttp://www.blogger.com/profile/10699612034298659706noreply@blogger.com9